Malignant salivary gland tumors of the paranasal sinuses and nasal cavity. The UCLA experience

Between 1962 and 1985, 35 patients with malignant salivary gland tumors of the paranasal sinuses and nasal cavity were treated with curative intent at UCLA. They were staged according to the American Joint Committee (TNM) classification for squamous cell carcinoma of the paranasal sinuses: 27 of 35 (77%) patients presented with T3-4 disease. Adenoid cystic carcinoma was the diagnosis in 24 (68%) patients, while adenocarcinoma accounted for half of the cases involving the ethmoid sinuses and nasal cavity. Eleven patients were treated with surgery alone, 13 with combined surgery and radiation, and 11 with radiation therapy alone. All patients were followed from 40 to 216 months. Analysis by treatment modality revealed a local control of 18% (2 of 11) in the surgery alone group, 62% (8 of 13) in the combined group, and 9% (1 of 11) in the radiation alone group. The group undergoing combined treatment achieved the highest local control rate despite having a higher proportion of patients with advanced stage and residual disease at the surgical margins. In patients with positive surgical margins, the tumor recurred locally in 4 of 6 (67%) unirradiated patients, compared with 3 of 10 (30%) of those undergoing postoperative irradiation. Patients with adenoid cystic carcinoma had poorer results, with only 25% remaining relapse-free, compared with 45% of patients with adenocarcinoma. The 5-, 10-, 15-, and 20-year actuarial survival for all cases was 73%, 60%, 32%, and 20%, respectively. We conclude that post-operative radiation improves the results of treatment in all patients with malignant salivary gland tumors in this location, compared with the results of surgery alone.     

The use of radiation therapy in the management of minor salivary gland tumors

Between 1964 and 1985, 52 patients were treated with curative intent by radiation therapy alone or in combination with surgery for malignant tumors of minor salivary gland origin. All patients had a minimum follow-up of 2 years, and 80% had a minimum follow-up of 5 years. Twenty-six (50%) were adenoid cystic carcinomas; the remaining histologies included adenocarcinoma, mucoepidermoid carcinoma, and malignant mixed tumors. The most common sites of origin were in the oral cavity/oropharynx (49%) and the nasal cavity or paranasal sinuses (40%). Twenty-seven patients (52%) presented with an advanced or unresectable stage (AJCC Stage III or IV, extensive bone or nerve invasion, or tumor greater than 5 cm). Treatment was highly individualized; 50% of the patients received radiation therapy alone, and 50% received combined treatment with either postoperative or preoperative radiation therapy. Early-stage minor salivary gland tumors were controlled equally well with radiation therapy alone or with a combined approach. For the advanced tumors, a combined approach yielded significantly superior absolute local control rates as compared with radiation therapy alone (10/13 vs. 2/13). For adenoid cystic carcinoma, the local control rate at 10 years was 45% (actuarial); the tumor was not controlled locally in any patients with advanced/unresectable stage who were treated with radiation therapy alone. The absolute local control rate was 75% for 4 early-stage tumors treated with radiation therapy alone and 60% for 5 advanced tumors treated with a combined approach. The average time to local recurrence was 67 months for adenoid cystic carcinoma. Severe complications of radiation therapy occurred in 11 (27%) of 40 evaluable patients, with unilateral blindness being the most common. Seven of 9 patients who became blind had unresectable disease with close proximity to or invasion of the orbit. A time-dose analysis is also presented.     

Postoperative irradiation of minor salivary gland malignancies of the head and neck.

OBJECTIVES: (1) To review the Stanford experience with postoperative radiotherapy for minor salivary gland carcinomas of the head and neck. (2) To identify patterns of failure and prognostic factors for these tumors. MATERIALS AND METHODS: Fifty-four patients with localized tumors were treated with curative intent at Stanford University between 1966 and 1995. The 1992 AJCC staging for squamous cell carcinomas was used to retrospectively stage these patients. Thirteen percent had stage I, 22% stage II, 26% stage III, and 39% stage IV neoplasms. Thirty-two patients (59%) had adenoid cystic carcinoma, 15 (28%) had adenocarcinoma, and seven (13%) had mucoepidermoid carcinoma. Thirty (55%) had positive surgical margins and seven (13%) had cervical lymph node involvement at diagnosis. The median follow-up for alive patients was 7.8 years (range: 25 months-28.9 years). RESULTS: The 5- and 10-year actuarial local control rates were 91 and 88%, respectively. Advanced T-stage (T3-4), involved surgical margins, adenocarcinoma histology, and sinonasal and oropharyngeal primaries were associated with poorer local control. The 5- and 10-year actuarial freedom from distant metastasis were 86 and 81%, respectively. Advanced T-stage (T3-4), lymph node involvement at diagnosis, adenoid cystic and high-grade mucoepidermoid histology were associated with a higher risk of distant metastases. The 10-year cause-specific survival (CSS) and overall survival (OS) were 81 % and 63%, respectively. On multivariate analysis, prognostic factors affecting survival were T-stage (favoring T1-2), and N-stage (favoring NO). When T- and N-stage were combined to form the AJCC stage, the latter became the most significant factor for survival. The 10-year OS was 86% for stage I-II vs. 52% for stage III-IV tumors. Late treatment-related toxicity was low (3/54); most complications were mild and no cranial nerve damage was noted. CONCLUSIONS: Surgical resection and carefully planned post-operative radiation therapy for minor salivary gland tumors is well tolerated and effective with high local control rates. AJCC stage was the most significant predictor for survival and should be used for staging minor salivary gland carcinomas.     

Management of salivary gland tumors

Surgery after proper imaging (MRI or CT scan) is the main stay of treatment for salivary gland tumors. Although excision margins should be ≥5 mm for malignant tumors in cases of parotid gland carcinoma, the facial nerve should be preserved whenever it is not infiltrated. Adjuvant external radiation is indicated for malignant tumors with high-risk features such as close (or invaded) margins, perineural speed, lymphatic and/or vascular invasion, lymph-node involvement and high-grade histology. A Phase II trial testing adjuvant concomitant cisplatin plus radiation therapy versus adjuvant radiation therapy alone after surgery is currently under investigation for high-risk salivary gland cancer. For inoperable cancers, photons combined with proton boost seem to be a valuable option. Even if protons or carbon ions are promising, access to the latter is limited for usual treatment. For recurrent and/or metastatic cancer, polychemotherapy (cisplatin based) gives a 25% response rate in adenoid cystic carcinoma and should be used when the disease is overtly in progression. Targeted therapies with anti-EGF receptor molecules, antiangiogenic agents and tyrosine kinase inhibitors are ongoing, but more trials are needed to establish their efficacy, as is the use of bortezomib followed by doxorubicin. The products of fusion oncogenes, which have a pathogenic role in some adenoid cystic carcinoma and mucoepidermoid carcinomas, are of interest as potential therapeutic targets.     

Desmoid tumor: prognostic factors and outcome after surgery, radiation therapy, or combined surgery and radiation therapy.

PURPOSE: To evaluate the therapeutic value of resection and the potential benefits of and indications for adjuvant and definitive radiation therapy for desmoid tumors. MATERIALS AND METHODS: We performed a retrospective review of 189 consecutive cases of desmoid tumor treated with surgical resection, resection and radiation therapy, or radiation therapy alone. Treatment was surgery alone in 122 cases, surgery and radiation therapy in 46, and radiation therapy alone in 21. Median follow-up was 9.4 years. RESULTS: Overall, 5- and 10-year actuarial relapse rates were 30% and 33%, respectively. Uncorrected survival rates were 96%, 92%, and 87% at 5, 10, and 15 years, respectively. For the patients treated with surgery, the actuarial relapse rates were 34% and 38% at 5 and 10 years, respectively. Among 78 patients with negative margins, the 10-year recurrence rate was 27%, whereas 40 margin-positive patients had a 10-year relapse rate of 54% (P = .003). Tumors located in an extremity also had a poorer prognosis than did those in the trunk. For patients treated with radiation therapy for gross disease, the 10-year actuarial relapse rate was 24%. For patients treated with combined resection and radiation therapy, the 10-year actuarial relapse rate was 25%. The addition of radiation therapy offset the adverse impact of positive margins seen in the surgical group. CONCLUSION: Wide local excision with negative pathologic margins is the treatment of choice for most desmoid tumors. Function-sparing resection is appropriate because adjuvant radiation therapy can offset the adverse impact of positive margins. Unresectable disease should be treated with definitive radiation therapy.     

Results of carbon ion radiotherapy in 152 patients

PURPOSE: This study summarizes the experience with raster scanned carbon ion radiation therapy (RT) at the Gesellschaft für Schwerionenforschung (GSI), Darmstadt, Germany since 1997. METHODS AND MATERIALS: Between December 1997 and December 2002, 152 patients were treated at GSI with carbon ion RT. Eighty-seven patients with chordomas and low-grade chondrosarcomas of the skull base received carbon ion RT alone (median dose 60 GyE); 21 patients with unfavorable adenoid cystic carcinomas and 17 patients with spinal (n = 9) and sacrococcygeal (n = 8) chordomas and chondrosarcomas were treated with combined photon and carbon ion RT. Twelve patients received reirradiation with carbon ions with or without photon RT for recurrent tumors. Furthermore, 15 patients with skull base tumors other than chordoma and low-grade chondrosarcoma were treated with carbon ions. RESULTS: Actuarial 3-year local control was 81% for chordomas, 100% for chondrosarcomas, and 62% for adenoid cystic carcinomas. Local control was obtained in 15/17 patients with spinal (8/9) and sacral (7/8) chordomas or chondrosarcomas and in 11/15 patients with skull base tumors other than chordomas and low-grade chondrosarcomas, respectively. Six of 12 patients who received reirradiation are still alive without signs of tumor progression. Common Toxicity Criteria Grade 4 or Grade 5 toxicity was not observed. CONCLUSION: Carbon ion therapy is safe with respect to toxicity and offers high local control rates for skull base tumors such as chordomas, low-grade chondrosarcomas, and unfavorable adenoid cystic carcinomas.     

The role of radiation therapy in squamous cell carcinoma of the upper aerodigestive tract with positive surgical margins

One hundred four patients with squamous cell carcinoma of the upper aerodigestive tract and with histologically positive surgical margins were evaluated for this historically controlled study to determine the efficacy of postoperative radiation therapy. Positive margins were defined as the presence of one or more of the following: carcinoma in situ, tumor within 0.5 cm from the surgical margins, microscopic disease, or dysplasia. Patients received either surgery alone (44 cases) or surgery plus postoperative radiation therapy (60 cases) and were followed for a minimum of 2 years. Treatment strategies, stage by stage, were similar for all patients. Surgery varied from simple excision in T1 to composite resection and/or laryngopharyngectomy with radical neck dissection in advanced cases. Radiation therapy was given postoperatively with doses ranging from 4,500 to 7,500 cGy. The overall 2-year survival rate with no evidence of disease (NED) was consistently higher in the surgery plus radiation therapy group. Furthermore, when the subgroup of patients who had dysplasia at the surgical margins was excluded from the analysis, the 2-year NED survival rate difference became statistically significant (p = 0.05). This outcome favored combined therapy (36 of 58 patients) over surgery alone (13 of 32 patients). This favorable result was obtained despite the high percentage of T3-T4 stages (79 vs. 21%) and clinically positive lymph nodes (83 vs. 17%) in patients who had received postoperative radiation therapy. The significance of dysplasia at the surgical margins and the impact of radiation therapy on the tumor and nodal control in this group of patients needs further clarification.     

Utility of surgical margins in the radiotherapeutic management of soft tissue sarcomas

Seventy-four adult patients with localized soft tissue sarcomas were treated with radiation therapy following surgery between 1965 and 1988. Fifty-three were treated after the first excision of their tumor with 6 (11.3%) local recurrences. Twenty-one received radiation after excision of recurrent disease with 11 (52.4%) local failures (P less than .0005). Metastatic disease occurred in 14 (26.4%) of the primary tumors and 8 (38.1%) with multiple previous excisions (P less than .48). Of those patients treated for primary sarcoma, there were no local failures with pathologically wide margins or if a single margin was microscopically positive. Local failure occurred in 4 of 26 (15.4%) if the tumor was merely enucleated and in 2 of 11 (18.2%) who had grossly positive surgical margins (P not significant). Local failure was also more common in truncal locations (33.3%) as compared with extremity locations (8.7%, P = .1359). Additional factors analyzed which adversely affected prognosis included tumor grade, stage, and inadequate radiation dose.     

头颈部腺样囊性癌放射治疗30例分析及文献复习

目的 :评价头颈部腺样囊性癌放射治疗的意义。方法 :回顾性分析 1971年 4月～ 1996年 4月收治的 30例头颈部腺样囊性癌的临床资料 ,生存情况并与文献比较。结果 :术后放疗 10年总生存率、10年无瘤生存率分别为 91 7%和 75 0 % ,首程放疗剂量 6 0～ 75Gy/ 6～ 7 5w ,5年内复发率 2 3%。结论 :术后放疗在本病治疗中占有很重要的地位 ,其可提高生存率 ,降低局部复发。     

Intraoral adenoid cystic carcinoma. The presence of perineural spread in relation to site, size, local extension, and metastatic spread in 22 cases

Twenty-two patients with an intraoral adenoid cystic carcinoma (ACC), initially treated by surgery with or without postoperative radiotherapy, were examined for the presence of perineural spread in relation to primary site, size, local extension, histologic status of the surgical margins, and metastatic spread of the tumor. There seems to be no correlation between perineural spread and the primary site or size of the tumor. However, perineural spread occurred more often in tumors with local extension and in cases with surgical margins with positive results. There seems to be no statistically significant correlation between perineural invasion and distant metastatic disease.     

Histologic grading of adenoid cystic carcinoma of the salivary glands

Seventy-nine patients with adenoid cystic carcinoma arising in salivary glands were studied to determine whether a correlation existed between the morphologic features of the tumor and the prognosis. Three histologic grades were established: Grade I, tumors with tubular and cribriform areas but without solid components; Grade II, cribriform tumors that were either pure or mixed with less than 30% of solid areas; and Grade III, tumors with a predominantly solid pattern. Cumulative survival rates at 15 years were 39%, 26%, and 5%, for Grades I, II, and III, respectively. Grade III tumors were larger, recurred frequently, and killed the patients within 4 years. Grade I lesions were smaller, were amenable to complete surgical excision, and had a protracted clinical course. Grade II tumors lay between the other two forms both clinically and pathologically. Other important prognostic features of the adenoid cystic carcinoma were its primary site, its presence or absence at surgical margins, and the anatomic structures it involved.     

Large, deep, high-grade extremity sarcomas: treating tumors of the flexor fossae

Limb salvage is now possible for the majority of patients with extremity sarcomas. Although overall prognosis is primarily based on tumor size and histologic grade, complete surgical excision and local control is essential for cure. There are, however, certain anatomic locations such as the flexor fossae in which a complete surgical margin is difficult to attain, and surgery without adjuvant therapy has a high local failure and amputation rate. We have found that preoperative adjuvant therapy consisting of chemotherapy and radiation followed by surgical excision with tumor-free margins has been successful in treating flexor fossa sarcomas with high limb salvage (96%), local control (89%) and overall survival rates (70%). These results are comparable to patients with similar large, high-grade extremity tumors in other compartmental locations.     

Postoperative irradiation in malignant tumors of submandibular gland

A retrospective review of 18 patients with treated primary malignant tumors of submandibular gland is presented. The data revealed that initial control of malignancies was inversely related to the presence of disease and surgical margins. Postoperative radiation therapy was used in cases with positive surgical margins. Of the patients with inadequate surgical margins, 71% achieved local relapse when they had surgery alone, compared with 27% of the group that received postoperative irradiation. This study suggests that adjuvant radiation therapy may improve the local control and survival. Such therapy can serve as the salvage method for those who have inadequate surgical margins. Re-excision is disappointing in its ability to control this disease.     

Carcinomas arising in the submandibular gland: high propensity for systemic failure

BACKGROUND: Cancers of the submandibular gland are uncommon and only a few small series have reported patient survival and prognosis. METHODS: We examined the treatment outcomes of 62 patients with surgically treated submandibular gland carcinomas. All patients underwent surgical excision with/without neck dissection, and 41 received postoperative radiotherapy for high-grade, invasive, positive margin, or regionally metastatic tumors. The locoregional control and survival rates were calculated by the Kaplan-Meier method and prognostic factors were calculated from uni- and multivariate analyses. RESULTS: Of the 62 submandibular gland carcinomas, 19 were adenoid cystic, 11 were mucoepidermoid, and 10 were salivary duct carcinomas, and 8 were carcinomas in pleomorphic adenoma. Actuarial 5-year locoregional control, distant metastasis-free survival, disease-free and overall survival rates were 69.7%, 65.8%, 52.8%, and 56.8%, respectively. In multivariate analysis, T category and histological grading were prognostic for disease-free survival (P < 0.01), and T category and resection margins were prognostic for locoregional control (P < 0.02). Distant metastases were found in 21 patients (33.9%) at initial staging (n = 2) or follow-up (n = 19). CONCLUSIONS: Despite effective locoregional treatment, approximately one-third of patients with submandibular gland carcinomas may fail systemically, resulting in poor survival. New, more effective therapies may be required for these patients.     

Postoperative radiotherapy for malignant fibrous histiocytoma.

Between 1974 and 1989, 49 patients with histologically confirmed malignant fibrous histiocytoma received postoperative radiotherapy at the Mallinckrodt Institute of Radiology for primary (41) or recurrent (8) disease. Median age of the patients was 63 years, and the median follow-up period was 41 months. Patients were grouped according to the 1988 AJC staging classification: stage IA (one patient), stage IIA (4 patients), stage IIB (9 patients), stage IIIA (15 patients), stage IIIB (18 patients), and stage IVA (2 patients). Eight tumors (16%) were in the pelvis, 8 (16%) in the trunk, 4 (8%) in the head and neck, and 29 (60%) in the extremities. Primary surgical procedures included incisional biopsy (4 patients), excisional biopsy (19), narrow margin excision (14), wide local excision (9), and removal of the entire compartment (3). Based on pathology reports, the margins of resection were classified as positive in 23 (5 gross, 18 microscopic), 5 close, 11 negative, and 10 unknown. Patients were irradiated with shrinking field technique; the median radiation dose was 6000 cGy, with more than 95% of patients receiving at least 4500 cGy. In addition, seven patients received postoperative chemotherapy. The 5-year overall survival rate was 62%, disease-free survival 64%, local control 68%, and freedom-from-distant metastasis 85%. Thirteen patients had local recurrences, with greater than 75% recurring within 3 years. Sites of local recurrence were as follows: trunk (3), pelvis (3), lower extremities (4), and head and neck (3). There appears to be a correlation of local failure with positive surgical margin: of 23 patients with positive margins, 9 (39%) had local recurrences, whereas 1 of 11 patients (9%) with negative margins had local recurrence. Three of 13 patients with persistent or recurrent disease were salvaged by additional treatment, rendering ultimate local control in 80% (39/49). Thirty-four of 36 patients with local control obtained good to excellent function. Two patients were found to have grade 3 complications: 1 patient had edema of the extremity, and the other developed necrotic skin ulcer that was successfully treated with hyperbaric oxygen. Five patients developed distant metastases, with 80% occurring within 2 years. In summary, adequate but conservative surgery with postoperative radiotherapy for malignant fibrous histiocytoma can achieve local tumor control as well as preservation of functional limbs with acceptable morbidity in a large proportion of patients.     

Malignant fibrous histiocytoma: a retrospective study of 109 cases

The purpose of this report is to assess the prognostic factors that could influence management and clinical outcome of malignant fibrous histiocytoma (MFH) of soft tissues. Between 1975 and 1998, 109 patients diagnosed with MFH of the soft tissues, seen at King Faisal Specialist Hospital and Research Center, have been reviewed. Of the 109 patients, 75 were men and 34 were women. The median age at presentation was 48 years (range: 3-94). Seven patients (6%) had regional nodal disease and 10 other patients (9%) with distant metastases were excluded from survival analysis. The remaining 92 patients had localized disease and had surgery as the primary treatment modality with or without radiotherapy and/or chemotherapy. Extremities were the most common location (58%). Tumors less than 5 cm represented 32%, whereas 68% had tumors 5 cm or more. Low-grade tumors constituted 46%, and the remaining 54% were high grade. Thirty-seven percent of patients had positive surgical margins histologically after complete gross resection. The 5- and 10-year relapse-free survival (RFS) rates were 39% and 36%, respectively. Isolated local recurrence occurred in 20 patients (22%), isolated metastatic disease without local recurrence in 9 patients (10%), and combined local and metastatic disease occurred in 20 patients (22%). The overall 5- and 10-year overall survival (OS) rates were 50% and 43%, respectively. On multivariate analysis, tumor size and radiation dose were significant factors for RFS (p = 0.04 and 0.0005, respectively). In terms of OS, size, histologic grade, and surgical margins were significant factors on multivariate analysis (p = 0.001. 0.006, and 0.0001, respectively). Complete surgical resection at the time of primary tumor presentation is likely to afford the best chance for RFS and OS. Radiation therapy plays an important role, in combination with surgery for better local control, particularly in high-grade lesions, and in cases with positive surgical margins after wide complete gross excision. The role of adjuvant chemotherapy remains investigational.     

Patterns of failure following local excision and local excision and postoperative radiation therapy for invasive rectal adenocarcinoma

The clinical course of 40 patients undergoing conservative surgical excision and 26 patients undergoing local excision and postoperative radiation therapy of rectal carcinoma was reviewed. Surgical procedures were transanal excision (55 patients), Kraske procedure (ten patients), and fulguration (one patient). The five-year actuarial survival, disease-free survival, and local control of all 66 patients were 70%, 77%, and 63%, respectively. For patients undergoing local excision alone, the prognostic features of lesion size greater than 3 cm, poorly differentiated histology, invasion into muscularis propria or deeper, moderate to marked stromal fibrosis, vascular or lymph vessel invasion, fragmented resection, and positive resection margins were associated with a local failure rate of 20% or greater. Of the 26 patients receiving postoperative radiation therapy, four patients have developed local failure. For subgroups of patients with small rectal carcinomas confined to the mucosa, local excision may be a reasonable alternative to abdominoperineal resection. For tumors with deeper invasion but limited to the bowel wall, local excision plus pelvic irradiation can be offered to preserve anorectal function.     

Adenoid cystic carcinoma of the head and neck treated by surgery with or without postoperative radiation therapy: prognostic features of recurrence

PURPOSE: This study sought to review a single-institution experience with the management of adenoid cystic carcinoma of the head and neck. METHODS AND MATERIALS: Between 1960 and 2004, 140 patients with adenoid cystic carcinoma of the head and neck were treated with definitive surgery. Ninety patients (64%) received postoperative radiation to a median dose of 64 Gy (range, 54-71 Gy). Distribution of T stage was: 26% T1, 28% T2, 20% T3, and 26% T4. Seventy-eight patients (56%) had microscopically positive margins. Median follow-up was 66 months (range, 7-267 months). RESULTS: The 5- and 10-year rate estimates of local control were 88% and 77%, respectively. A Cox proportional hazards model identified T4 disease (p = 0.0001), perineural invasion (p = 0.008), omission of postoperative radiation (p = 0.007), and major nerve involvement (p = 0.02) as independent predictors of local recurrence. Radiation dose lower than 60 Gy (p = 0.0004), T4 disease (p = 0.005), and major nerve involvement (p = 0.02) were predictors of local recurrence among those treated with surgery and postoperative radiation. The 10-year overall survival and distant metastasis-free survival were 64% and 66%, respectively. CONCLUSION: Combined-modality therapy with surgery followed by radiation to doses in excess of 60 Gy should be considered the standard of care for adenoid cystic carcinoma of the head and neck.     

Adenoid cystic carcinoma of the nasopharynx: a case report]

Nasopharyngeal adenoid cystic carcinoma is a rare tumor, few cases have been reported in the literature. The aim of this report is to present a new case of an adenoid cystic carcinoma of the nasopharynx. We report the case of a 50 years-old woman presenting with an adenoid cystic carcinoma of the nasopharynx with diffuse pulmonary metastasis. The treatment combined chemotherapy, based on cisplatin and adriamycin drugs, and palliative radiation therapy at the dose of 30 Gy. The patient died 6 months after diagnosis establishment. The best treatment of adenoid cystic carcinoma is complete surgical resection but extended resection can be difficult in some locations as nasopharynx. The adjuvant radiation therapy seems to improve local control.     

Sinonasal adenoid cystic carcinoma: the M. D. Anderson Cancer Center experience

BACKGROUND: Adenoid cystic carcinoma of the sinonasal tract is a rare cancer that accounts for 10% of all malignancies at this site. The objective of the current study was to evaluate prognostic factors, treatment outcomes, recurrence patterns, and survival rates for sinonasal adenoid cystic carcinoma. METHODS: A retrospective chart review was performed at an academic tertiary referral center. Between 1990 and 2004, 105 patients were evaluated for adenoid cystic carcinoma of the sinonasal tract at a single institution. Demographics, presentation, anatomic site, Tumor, Lymph Node, Metastasis (TNM) classification, pathology, treatment, recurrences, and survival were evaluated. RESULTS: One hundred five patients with adenoid cystic carcinoma were evaluated, including 58 women and 47 men. Their median age was 50 years, and the mean follow-up was 47 months. The maxillary sinus (47%) and the nasal cavity (30%) were the most common primary tumor sites. The majority of patients presented with T3/T4 (76.7%), N0 (98%), M0 (97%) disease. Eighty-four percent of patients underwent surgery and received postoperative radiation as treatment for their primary disease. The local recurrence rate was 30%, and the distant metastases rate was 38%. The 5-year overall survival and disease specific survival rates were 62.9% and 70.9%, respectively. CONCLUSIONS: Adenoid cystic carcinoma of the paranasal sinuses is a rare disease, and the ideal treatment paradigm has yet to be defined. The current data suggested that surgical resection with postoperative radiation therapy offers durable local control and compares favorably with historic data. Although local recurrences develop in a significant percentage of patients, survival from this disease exceeds that of other sinonasal malignancies.